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Definition uncontrolled clonal proliferation of myeloid cells a myeloproliferative disorder
Epidemiology 40-60y/o M>F
History chronic & insidious ~30% detected by chance weight loss, fatigue, sweats, fever features of:1. gout d/t purine breakdown2. bleeding d/t platelet dysfunction3. abdominal discomfort d/t splenomeagly
Physical Examination splenomegaly (>75%) hepatomegaly, anemia, bruising
Investigation results WCC markedly elevated- neutrophils- myelocytes- basophils- eosinophils Hb low or normal platelets variable bone marrow hypercellular Ph on cytogenetics (blood or bone marrow)
Prognosis median survival 5-6 years 3 phases:1. Chronic - lasts months to years, few Sx2. Accelerated - increasing Sx, spleen size, difficulty controlling counts3. Blast - features of acute leukemia +/- death
Treatment tyrosine kinase inhibitor if Ph+ (eg. imatinib) allogeneic stem cell transplant from HLA-matched sibling / unrelated donor- 1st line only in young patients a-interferon (+/- cytarabine for chronic phase patients
CML
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